摘要目的 探讨成人髓母细胞瘤(MB)不同病理学亚型的临床特点及预后.方法 回顾性分析2000年6月至2012年6月151例成人MB的临床资料、影像学和病理学表现,采用x2检验对各亚型临床特征进行分析,Kaplan-Meier生存分析对各亚型生存期进行比较.结果 151例MB包括经典型73例,促纤维增生/结节型36例,间变型39例,大细胞型3例;其中促纤维增生/结节型较其他亚型更常见于小脑半球(P=0.000),间变型和大细胞型较其他亚型更容易复发(P=0.000)及转移(P=0.003);至随访期结束无瘤生存患者91例,带瘤生存15例,死亡45例,生存时间为6 ～ 150个月,平均生存时间(103.3±5.7)个月(95％ CI 92.52 ～ 115.09);其中经典型组、促纤维增生/结节型组、间变型和大细胞型组平均生存期分别为(110.7 ±7.8)、(125.5 ±7.6)和(57.6±7.6)个月,差异具有统计学意义(P =0.000).结论 成人MB各个病理亚型具有不同临床特点,而间变型和大细胞型成人MB患者预后不良.

abstractsObjective To study the pathologic characteristics and prognosis in different subtypes of adult medulloblastoma (MB).Methods The clinical information,imaging findings and pathologic characteristics of 151 cases of adult medulloblastomas were retrospectively reviewed and analyzed by chi-square test.The survival data were assessed using the Kaplan-Meier method.Results Amongst the 151 MB cases studied,there were 73 cases of classic MB,36 cases of desmoplastic/nodular MB,39 cases of anaplastic MB and 3 cases of large cell MB.The primary tumors were more frequently located in cerebral hemisphere in desmoplastic/nodular MB than in other subtypes (P =0.000).On the other hand,large cell/ anaplastic MB were associated with more frequently local recurrence and distant metastasis (P =0.003).The post-operative overall survival time ranged from 6 to 150 months,with median survival being (103.3 ± 5.7) months (95％ CI,92.52 to 115.09).The median survival of classic MB,desmoplastic/nodular MB and large cell/anaplastic MB was (110.7 ± 7.8) months,(125.5 ± 7.6) months and (57.6 ± 7.6) months,respectively.The differences were statistically significant (P =0.000).Conclusions The three variants of MB show different biologic behavior.Large cell/anaplastic MB represents an independent poor prognostic indicator in adults.